Table of Contents
Definition / general | Sites | Pathophysiology | Clinical features | Radiology images | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Immunohistochemistry & special stains | Differential diagnosisCite this page: Tsang P. Angiolipomatous hamartoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphnodesangiolipomatoushyperplasia.html. Accessed December 21st, 2024.
Definition / general
- Benign mesenchymal proliferation with vascular and fatty components (Pathologica 2006;98:239)
- Associated with Castleman lymphadenopathy, hyaline vascular variant (Arch Pathol Lab Med 1986;110:853)
Sites
- Various nodal sites, including cervical, mediastinal and retroperitoneal lymph nodes
- Occurs in nodal parenchyma or extracapsular soft tissue
- Miscellaneous extranodal sites can be involved
Pathophysiology
- Rare disorder of unknown etiology
- Pseudotumor; not a true neoplasm
Clinical features
- Innocuous and slow growing
Case reports
- 16 year old boy with paraparesis caused by an angiolipomatous hamartoma with Proteus syndrome and scoliosis (J Neurosurg 2005;103:282)
- 60 year old man with a spindle cell sarcoma containing a focal angiolipomatous hamartoma component (J Pathol 2002;197:264)
- 70 year old man with angiolipomatous mesenchymal hamartoma (angiolipomatosis) of sigmoid mesocolon (Int J Clin Exp Pathol 2011;4:210)
- Giant lymph node hyperplasia in an angiolipomatous mediastinal mass (Arch Pathol Lab Med 1986;110:853)
- Vascular transformation of sinuses in bilateral cervical lymph nodes (Head Neck 1999;21:366)
Treatment
- Surgical excision
Gross description
- Unencapsulated single or multiple yellow and fatty nodules up to 15 cm
- Tan nodules may represent accompanying hyaline vascular Castleman lymphadenopathy
Microscopic (histologic) description
- Noncircumscribed intranodal or extranodal mass
- Mature adipose tissue and haphazard thick walled muscular vessels of varying sizes
- No thrombi within disorganized vascular channels
- Lacks other mesenchymal tissue types, such as muscle
- No necrosis, dense sclerosis, lipoblasts or marked cytologic atypia
Immunohistochemistry & special stains
- Smooth muscle actin and desmin highlight muscular vessels
Differential diagnosis
- Angiomyolipoma: contains myomatous component, common in kidney
- Lipofibromatosis: pediatric soft tissue tumor with conspicuous fibrous component
- Liposarcoma: malignant tumor with lipoblasts